Poromatosis Following Ewing SarcomaAmr Abduljabbar1*, Omar Alanazi2, Amany Fathadin3 and Mohammad Alhaddab2

1Department of Dermatology, King Saud Medical City, Riyadh, Saudi Arabia2Department of Dermatology, King Saud University Hospital, Riyadh, Saudi Arabia3Department of Pathology, King Saud University Hospital, Riyadh, Saudi Arabia*Corresponding author: Amr Abduljabbar, Department of Dermatology, King Saud Medical City, Riyadh, Saudi Arabia, Tel: +966566477766; E-mail: Amr8002@hotmail.com

Received date: June 21, 2016; Accepted date: August 09, 2016; Published date: August 11, 2016

Copyright: © 2016 Abduljabbar A, et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permitsunrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.

Abstract

Eccrine poromas are rare, benign adnexal tumors derived from the intraepidermal portion of sweat ducts.Historically they were thought to arise from eccrine ducts although today it is thought that they may also have anapocrine origin. They usually appear as solitary, slow-growing, skin-colored papules on acral surfaces. Here wepresent the unusual situation of a patient with multiple poromas who was previously treated with chemotherapy andradiation for Ewing sarcoma. This report adds to the increasing evidence that connect multiple poromas to sometreatments of malignant conditions.

Keywords: Ewing sarcoma; Poroma; Poromatosis

IntroductionA poroma is a benign adnexal neoplasm composed of epithelial cells

that show tubular differentiation (usually distal ductal), whereas aporocarcinoma is a malignant poroma which can cause visceralmetastasis [1]. Historically poromas were considered as glandularadnexal neoplasms of eccrine lineage, and that is why it was referred toas eccrine poroma. However, nowadays it has been proved that thisdescription was not accurate and poromas can be of apocrine lineagetoo [2]. Multiple poromas or eccrine poromatosis is an uncommonphenomenon and more than half of the reported cases had a history ofimmunosuppression from either radiation or chemotherapy [3]. Herewe present a 51-year-old gentleman with eccrine poromatosis, who wasalso treated with chemotherapy and radiation before the onset of thelesions.

Case ReportA 51-years-old Saudi male with a history of Ewing sarcoma of the

chest, which was diagnosed in April 2012 in the university hospital. Hepresented initially with 3 months history of painless graduallyenlarging right chest mass. No other complaints and systemic reviewwas unremarkable. Blood studies were within normal parameters. Achest X-ray showed opacity in the right side of the chest. Computedtomography was ordered for further delineation of the opacity andrevealed a large well defined lobulated heterogeneous solid enhancingmass with central low density area measuring 5.1 x 6.6 cm lying

subcutaneously in the right anterior chest wall and reaching the lowerlevel of sternum. There was no evidence of mediastina or otheradenopathies. A diagnosis of primitive neuroectodermal tumor(PNET) of the chest wall was made through a CT-guided biopsy of themass. An isotope bone scan confirmed that no other skeletal site wasinvolved. The patient underwent surgery on May 3, 2012, in form ofwide local excision of the right chest wall mass and rhomboid flap.Histopathology of the mass showed no evidence of the neoplasmextension to the right chest wall muscle. The tumor was classified asstage 1b. After the surgery, fractionated radiotherapy of 45 Gy wasdecided, but the patient was not able to continue for social reasonsafter receiving two sessions. Chemotherapy has been offered and hefound it more convenient for his situation. He received a regimenconsisting of Etoposide, Ifosfamide and Mesna starting on June 5,2012. He was given 4 cycles of chemotherapy with the last one onOctober 19, 2012. A repeat CT scan did not show any recurrences. Todate, the patient remained disease-free after 4 years of follow-up in theoncology clinic. The patient was seen at the dermatology clinic in July2015 because of eruptive multiple papules and nodules on the chestand abdomen. These lesions had developed over the past year and areasymptomatic. On examination the patient looks fine, although he hasa long scar from his previous surgery over the right chest and flank. Hehas multiple erythematous to brown, mildly firm papules and nodulesover the anterior trunk ranging in size between 0.3 to 1 cm (Figures 1aand 1b) involving the chest and abdomen. The back, extremities andother body sites are free of such lesions. A review of systems wasunremarkable.

Abduljabbar et al., J Clin Exp Dermatol Res 2016, 7:5

DOI: 10.4172/2155-9554.1000366

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Figure 1: (a) Multiple erythematous to slightly brown firm papules and nodules over the anterior trunk; (b) closer view of the poromas on thechest.

A biopsy was taken and histological analysis revealed well-circumscribed tumors composed of nests and cords of proliferatingbasaloid cells. The cells were uniform and smaller than the epidermalcells with which they were in contact. The stroma was vascular andducts were seen in the tumor nests (Figures 2a-2c). Based on thosefeatures, a diagnosis of multiple poromas was made. All the lesions

were excised and sent for pathology, and sections from all lesionsconfirmed the same diagnosis. There was no distinctivehistopathological feature differentiating these poromas in our patientfrom the typical de novo lesions. Patient was seen after 1 year of lesionsremoval with no recurrences or new lesions.

Figure 2: (a) Biopsy of one of the nodules shows circumscribed tumors composed of nests of basloid cells extending downward from theepidermis. Hematoxylin and eosin x40; (b) Medium magnification of the tumor shows uniform small cells and vascular stroma. Hematoxylin& eosin x100; (c) Ducts present in the tumor. Hematoxylin & eosin x400.

DiscussionEccrine poroma (EP) is a benign, mostly asymptomatic, slow

growing or stable neoplasm appearing as nodular lesions, occasionallypigmented, which occurs typically on the palms and soles [4]. Itspathogenesis remains unclear. The earlier literature explains that by thehigh concentration of eccrine glands on the acral surfaces, especiallythe sole of the foot. However, newer reports demonstrate that it canform elsewhere on the body, including areas without a high density ofeccrine ducts and in hair-bearing areas [5,6]. It arises within theepidermis and it is superficial, with sharply demarcated borders,originating from the uppermost portion of the intra-epidermal eccrine

duct and the acrosyringium [7]. As the lesion develops, the tumorstarts to develop in the dermis layer. Poromas usually present assolitary papules, plaques or nodules and it can appear on anycutaneous surface, where they are less readily specifically identified.Another common site is the scalp, with or without an associated nevussebaceous. EPs have been reported in all age groups, including children[5,8]. Poromas commonly display highly vascularized stroma, and aclinical pattern suggestive of pyogenic granuloma is common,especially in poromas that involve acral skin. Due to their benignnature treatment can be optional, although some clinicians emphasizethe importance of excision to avoid possible future malignancy [2].Superficial lesions may be treated by shave or electrosurgical

Citation: Abduljabbar A, Alanazi O, Fathadin A, Alhaddab M (2016) Poromatosis Following Ewing Sarcoma. J Clin Exp Dermatol Res 7: 366.doi:10.4172/2155-9554.1000366

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destruction. Superficial or deeper lesions may also be treated by simpleexcision.

In histologic examination, poroma consists of a circumscribedproliferation of small, compact, pale cuboidal epithelial cells withmonomorphous nuclei and scant eosinophilic cytoplasm. The pale-staining aspect of these cells distinguishes them from the dark-stainingcells of basal cell carcinoma (BCC). Poromas can be wholly intra-epidermal, a pattern known historically as hidroacanthoma simplex;can occur in nests and cords in the dermis with broad continuity withthe epidermis and are then known as juxtaepidermal poroma, or theycan develop wholly within the dermis and are known as intradermalporoma, a pattern known historically as a dermal duct tumor.

Poromas may occasionally display tubular foci in which a centralcuticle is lacking. If such tubular foci are lined by columnar cells withholocrine secretion at the luminal border, a presumptive diagnosis ofapocrine poroma can be offered [4]. Many other evidences nowsuggest that some of these lesions may actually be derived from theapocrine gland rather than the eccrine gland [2].

Although poromas can usually be deemed benign, based oncircumscription and benign cytologic features, these lesions notuncommonly show small foci of necrosis en masse and a highlyvascularized (granulation tissue–like) stroma, the combination ofwhich can create a concern for malignancy, especially in inexperiencedobservers.

In rare reports, multiple poromas can develop, either in an acrallocation or in a widespread distribution, a clinical pattern referred to asporomatosis. One old report in 1970 described an acral type ofporomatosis but did not mention if that patient has any underlingmedical problems that could be related [9] Wilkinson et al. described apatient with hidrotic ectodermal dysplasia presented with diffuseporomatosis involving the trunk and extremities, including palms andsoles [10]. Four cases of eccrine poromatosis have been reported inassociation with radiotherapy or chemotherapy in patients diagnosedwith different kinds of malignancies including osteomyelitis, mycosisfungoides, acute lymphocytic leukemia and non-Hodgkin’s lymphoma[5,11-13].

In another recent paper, three patients were reported with non-Hodgkin’s lymphoma and one with malignant fibrous histiocytosis,who were all treated with chemotherapy and presented after variousperiods of time with multiple poromas [14]. They stated thatchemotherapy is a well-documented cause of various cutaneousdisorders, such as neutrophilic eccrine hidradenitis (NEH) andsyringosquamous metaplasia, probably due to the concentration ofchemotherapeutic agents or their metabolites in the sweat glands andducts as described in the literature before. They noticed long latencyperiods between the end of chemotherapy and the diagnosis of eccrineporoma and suggested the possibility that the development of eccrineporoma is associated with remodeling of the sweat apparatus or theregeneration of damaged skin appendages.

In 2013, as reported in the American journal of Dermatopathology,a 72-year-old woman underwent radical excision of anadenocarcinoma of the right nasolacrimal duct and eruptive poromasoccurred soon after radiation therapy. The patient was not treated withchemotherapy. They concluded that the occurrence of multipleporomas may be related to immunosuppression [15].

In our report, the presentation started almost 3 years after thechemotherapy and radiotherapy but still we think it is related to them,

as longer latency periods were the rule in most of the previous reports.It was hard to know which of them could be the most responsible asboth modalities were used in our patient, and even harder to knowwhich single chemotherapeutic agent most likely related todevelopment of poromas. Patient withdrawal from radiotherapy afteronly two sessions could raises more suspicion toward thechemotherapy. Although the lesions developed mostly nearby butoutside the field of radiotherapy, literature has described eruptiveporomas after radiotherapy both at the irradiated field and in non-treated areas [12,15]. After reviewing the cases reported associationwith chemotherapy, ifosfamide and its parent analoguecyclophosphamide have been mentioned in 6 cases including our case,while doxorubicin and its derivative epirubicin in 5 cases. Although itis difficult to assert their causality, cyclophosphamide and doxorubicinwould be the most suspected agents associated with poromas.Vincristine has been mentioned in 4 cases [5,14]. Another casereported association with total body irradiation in patient withleukemia also treated with chemotherapy, but the chemothraputicagents were not mentioned in this article [13].

The prognosis of poromas is favorable and even poromatosis is notknown to be associated with other anomalies. However, given therarity of these neoplasms, there remains a paucity of information onatypical presentations, recurrence and rates of malignanttransformation. Eccrine porocarcinoma may arise de novo or maydevelop from a pre-existing poroma. The variation in the reportedrates of malignant transformation from benign poromas is veryevident. It has been reported to be as high as 100%, while othersreported only 18% of poromas become malignant. The progression tomalignancy takes a mean of 8.5 years [16]. If the lesion recurs afterexcision or pre-existing poroma presents with spontaneous ulceration,bleeding, pain, sudden itching or accelerated growth, eccrineporocarcinoma should be suspected. It tends to appear more exophyticand ulcerative than a benign poroma. Our patient had no recurrencesor new lesions after 1 year of follow up and he will be under annualreassessment.

In conclusion, our report will add to the increasing evidence in theliterature that connect multiple poromas to some sort of treatments,especially for malignant conditions. Finally, these reports suggest thatchemotherapeutic agents and radiotherapy could be responsible for theoccurrence of the multiple eccrine poromas.

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Citation: Abduljabbar A, Alanazi O, Fathadin A, Alhaddab M (2016) Poromatosis Following Ewing Sarcoma. J Clin Exp Dermatol Res 7: 366.doi:10.4172/2155-9554.1000366

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Citation: Abduljabbar A, Alanazi O, Fathadin A, Alhaddab M (2016) Poromatosis Following Ewing Sarcoma. J Clin Exp Dermatol Res 7: 366.doi:10.4172/2155-9554.1000366

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